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Infantile spasms — RACP Paediatrics MCQ

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ModerateNeurologyInfantile spasmsRACP Paediatrics

A 6-month-old presents with clusters of brief flexion spasms occurring on waking. Each spasm lasts 1-2 seconds with a cry afterwards. Episodes occur in clusters of 10-20 over several minutes. Parents have noticed developmental regression. EEG shows hypsarrhythmia. What is the most likely diagnosis?

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Correct answer: A — Infantile spasms (West syndrome)

Clusters of brief flexor spasms on waking with developmental regression and hypsarrhythmia on EEG is diagnostic of infantile spasms (West syndrome). This is a neurological emergency. First-line treatment is vigabatrin (especially if tuberous sclerosis is suspected) or prednisolone/ACTH.

Reference: RCH Melbourne – 2023 – Infantile Spasms CPG; RACP Paediatric Curriculum – Neurology